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Cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement |
European Journal of Dermatology. Volume 1, Number 1, October 2008, Review article
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 Full Text
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Author(s) : Stephan Schreml, Rolf-Markus Szeimies, Thomas Vogt, Michael Landthaler, Josef Schroeder, Philipp Babilas |
Summary : Extracellular deposition of altered autologous protein (amyloid protein) within the dermis is the hallmark of cutaneous amyloidoses and systemic amyloidoses with cutaneous involvement. Amyloidoses may be acquired or hereditary in nature and subclassification differentiates between primary amyloidosis (no obvious predisposing disease) and secondary amyloidosis (specific underlying disease). More than 26 different proteins and peptides have been identified as amyloid precursors and these proteins are used to subclassify this heterogenous group of diseases. The amyloid proteins show an anti-parallel β-sheet conformation and form non-branching linear filaments of variable lengths and diameters of approximately 7.5 to 10 nm. However, the exact etiopathogenesis of amyloid formation still remains unclear. Depending on histoanatomical distribution and amount, amyloid may cause progressive and life-threatening organ dysfunction. Clinical presentation, histology, electron microscopy, and biochemical-immunological differentiation represent decisive tools for an accurate diagnosis. |
Keywords : amyloidosis, hereditary, non-hereditary |
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